title: Sclerosing rhabdomyosarcoma of a chest wall in an adult: a case report and review of the literature.
abstract: Sclerosing rhabdomyosarcoma (SRMS) is a newly recognized and rare variant of rhabdomyosarcoma. This soft tissue tumor has not yet been reported as a thoracic lesion. We report a case of a 26-year-old woman who presented with a large chest wall tumor. The tumor originated from the right anterior chest wall and protruded into the intra- and extrapleural cavity. A transcutaneous needle biopsy revealed spindle cells in an abundant hyalinized and fibrous stroma. Although the tumor was considered as a malignant soft-tissue neoplasm, a definitive diagnosis could not be established. A wide excision of the chest wall including the second, third and fourth rib and a part of sternum was performed. Histologically, cytoplasmic cross-striations were found in a portion of the tumor cells. The tumor cells were positive for muscle markers, and the tumor was diagnosed as rhabdomyosarcoma consistent with a sclerosing type of rhabdomyosarcoma. Eighteen months after the complete resection, the patient has pleural disseminations but is alive and undergoing chemotherapy. This case highlights the histologic features of a rare form of rhabdomyosarcoma, and emphasizes the importance of awareness of its existence and the utility of skeletal muscle markers in distinguishing sclerosing rhabdomyosarcoma from its mimics.
authors.affiliation: Department of Thoracic Surgery, Pathology, Mitsui Memorial Hospital, Chiyoda-ku, Tokyo, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Mikubo
authors.forename: Shingo
authors.initials: S
authors.lastname: Ikeda
authors.forename: Tatsuhiro
authors.initials: T
authors.lastname: Hoshino
authors.forename: Toshiya
authors.initials: T
authors.lastname: Yokota
authors.forename: Akiko
authors.initials: A
authors.lastname: Fujii
authors.forename: Masaya
authors.initials: M
authors.lastname: Mori
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D009361:Neoplasm Invasiveness; D010027:Osteotomy; D010997:Pleural Neoplasms / Q000188:drug therapy / Q000556:secondary*; D012208:Rhabdomyosarcoma / Q000737:chemistry / Q000556:secondary* / Q000601:surgery; D012598:Sclerosis; D056346:Sternotomy; D013899:Thoracic Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D035441:Thoracic Wall / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D013908:Thoracotomy; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D047368:Tumor Burden
chemical_list: D014408:Biomarkers, Tumor